Thalassemia Patients and Friends
Discussion Forums => Miscellaneous Questions => Topic started by: joyce on November 05, 2010, 02:12:28 PM
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my son diagnosed as having thal major when he is 3mths old ( september,2010 ). since then i will look any information regarding thal here and there. my really concerned is the enlarge spleen and liver issue. also im thinkin of my baby's physical growth. what can i do to make sure my child to have normal physical growth without enlarge spleen/liver?normal teeth/forehead/cheeckbones and skin colour? it is because from where i come from, it is a common view to see thallesemic with big stomach/forehead/ blue,pale skin. its really worried me. am afraid of my son will end up like one of that kind. as for my son is still toddler,i hope to see him grow as a normal kid.
please thalpal, guide me on this..
thnk you..
love-joyce. :rolleyes
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The most important thing you can to to ensure normal growth is to keep the hemoglobin level no lower than 9 before transfusions. With the availability of modern iron chelation, the recommendations for Hb level have changed to favor proper development over lower iron intake. Children who are kept at the proper Hb range, do grow much more normally and do not exhibit the bone deformations you have seen in some thal children.
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dear andy, thnks for tht reply but i do have another qustions.
my 1st son, 1yr 4 mths old..recently, i notice somting unusual with his appearnce. his skin around face/earlobe/ palm and feet look white/yellow. am not sure if it is pale. but his lips red as an usual, active with no problm with his appetite. i checked his spleen and liver-nothing unsual. he recently sleep late at night, around 11pm but wake up late too. never had flu/cold without cause. im worried for his brother as we have thal history in family.
we alrdy took him to have blood screen but there is no result so far. i just cant wait for the result as i've seen his younger brother suffered from thal. its unbearable..
andy, hope you can state some physical sign as a guide.
thanks!
love-joyce.
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Hi Joyce,
I can understand what you are feeling,i used to worry about my non thal son a lot,even after i got his electrophoresis report which said he didn't even have thal trait,but kids can look a bit pale sometimes,and since you are not sure he is pale,i hope there is no problem,thal majors are usually diagnosed with in initial months of their lives,like your second son,but its possible that your first son might have thal minor,but still its not necessary,if he is active as usual and have a good appetite,then don't worry :) although i know its easier said then done,keep us posted about the results.
Zaini.
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Thanks Zaini for understanding...
I also hope ther is no problm with my 1st son. Today i've checked thier HB. The result end up with my 1st 10.4Hb and his brother (Whic is thal major) 10.2Hb. I duno if my 1st son blood counting is normal for kids thier age (please anyone??) as for his brother, 23rd this month is his 3rd review. hope to see his blood maintain on 10.
While am at hospt, the nurse there askd if i hve started the desperal. she said it is better to start it as early as i could. she said, her nephew grew like other normal children without blue/pale skin and no bone deformations because they started the desperal while he's a year old.i wonder, when is the best time to start the iron chelation? can we jus start it before he done his 10th transfusion?
Pray to my sons dear friends..
Love-Joyce.
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Hi Joyce,
With an Hb over 10, it is likely your first son is thal minor. He may benefit from vitamins and supplements.
Chelation is now recommended earlier than it once was, but it is still somewhat early for your son. I think I would wait until after one year old before making this decision.
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Dear andy,
this is the result of my 1st son screening test.the doc said at the moment, the result is not confirm because his Hb F is high so hv to wait for another months for the blood to switch to adult. i wonder if you guys can help translating this as am not sure for what the doc explain ( actly he's just read the result by refering the google on 'haemoglobin electrophoresis' which i think am far more equipped with the info since i study on it here and there now :biggrin)..
Haemoglobin Electrophoresis pattern
cellulose acetate pH 8.6:
A marked increase in intensity is noted at the F region.
Quantitation of haemoglobins Normal range
Hb A2 (HPLC*) : 1.6 % ( 2.1 - 3.7 )
Hb F (HPLC*) : 108.0 % (less than 1.0 )
HLPC: markedly increased in Hb F with no Hb A is noted and Hb A2 of 1.6%
Peripheral blood Film:
Red blood cell morphology: Hb is within normal limit for age. Mild hypochromic microcytic RBC. Marked anisopoikilocytosis . Numerous target cells and misshapened red cells seen. Ocassional nRBC seen.
Others:
Haemoglobin concentration 11.3 g/dl
H inclusions Negeative (repeated)
Inttacellular Hb F distribution (kleihauer test) pancellular
Comment:
Thalassaemic full blood picture features with high Hb F.
Homozygous Delta Beta Thalassaemia. However, normaly patient will present with mild thalassaemia intermedia to severe thalassaemia major.
*Haemoglobin concentration 11.3 g/dl (is on 25/10/2010 but yesterday 09/11/2010 his Hb count is 10.4 g/dl)
please someone..
Love-joyce.
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Hi Joyce,
At what age was he when this test was done and what is his current age?
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hi andy,
the test was just taken last month -25/10/2010- at that time, he is 1yr 3mths.
love-joyce.
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Hi Joyce,
I don't know how much your doctor explained to you, but there is a complete absence of HbA, the normal type of hemoglobin. There is also a low amount of the secondary hemoglobin, HbA2, which indicates that the delta gene is also affected. The delta and beta genes are very close to each other on the same chromosome and the delta gene normally produces only a small amount of hemoglobin. However, in spite of having no normal hemoglobin, your son has an Hb of over 10 at this point in time. This is completely due to a very high level of fetal hemoglobin (HbF). This hemoglobin is what is used by the fetus and usually drops to very low percentages by age 6 months. The fact that there is enough of this HbF being produced at 15 months to have a hemoglobin in the thal minor range shows that fetal hemoglobin is still being produced and at a high rate.
Comment:
Thalassaemic full blood picture features with high Hb F.
Homozygous Delta Beta Thalassaemia. However, normaly patient will present with mild thalassaemia intermedia to severe thalassaemia major.
This boy also has thalassemia. Because there is a complete absence of HbA, there is no doubt that this is beta zero thalassemia, which under normal circumstances would result in transfusions at a very young age. But, there is an observation in this condition where the delta is also affected that there is normally a higher rate of HbF found than in beta zero thalassemia. This is possibly a result of increased gamma globin, which replaces beta globin in HbF, which is known to occur in the delta/beta zero thalassemia patients, and/or it may be the result of a condition that seems to have some connection to beta/delta thal. This condition is known as hereditary persistence of fetal hemoglobin (HPFH). In non-thals, this is also found with no real consequence, but when it is found in thals, it is a blessing, because it results in a higher production of HbF throughout life. Your son has a very high HbF level for a child his age. As the doctor told you, it may take some months before the full picture is clear, but I would have to say that there is an unusual high production of HbF at this age and this is most likely due to one of the two factors I mentioned, or both, and that this will result in a less severe, intermedia condition. He may be able to avoid transfusions, but if eventually does need them, it may be less frequent than in a major.
I know this isn't what you hoped to hear, but I do want you to understand that even though he does have thal, it is very likely not severe. There is also a good chance that his hemoglobin level can be increased through fetal hemoglobin inducing supplements like wheatgrass and resveratrol and possibly by the drug, hydroxyurea, if the Hb drops below 8. A good diet and vitamins without iron can also be beneficial.
Right now, you wait and see how things progress and if he can maintain a hemoglobin level high enough to avoid transfusion. I would highly suggest he be put on a baby vitamin without iron and also takes folic acid. These should also be taken by the infant. Do you have the test results for the youngest child also? I would like to see the results of his electrophoresis and any comments, if these are available, so I can compare them to the older boy's results. I especially would like to know if there is any mention of delta in the younger boy's results. His HbF will be high because of his young age, so that won't tell us much at this point, but it would be helpful to see any comments. Is the younger boy transfusing yet?
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Hi andy..
I've studied the reply you've posted. read it many times before i came to the conclusion that -whatever may happen next, i have to be strong! For know, i only can wait and monitoring my children diet. I gave him supplement(chewable appeton lysine) and goatmilk (purenat). He's doing fine, but look pale a bit espcly at his palm/feet and ears. hopefully he'll maintain his hemoglobin level up to 10. can i gve him folic acid , 1 mg?(-the one that his younger brother have know.) what supplement/ vitamin can i gv him to help inducing his HbF?
I already have my youngest's child result but haven't photocopied it yet ( stil at the doc's file, this coming 23rd is our appointment to see her.) as soon as i got it , i'll post it here.
Thanks andy!
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Ho Joyce,
At their ages, they can take folic acid, natural vitamin E (100 IU daily) and I would also recommend liquid vitamin D. When they're a bit older, I would suggest adding a daily B-complex and also a supplement like Osteocare for minerals. At their current age, wheatgrass liquid extract would be most likely the only way you can give wheatgrass. Later on you may be able to give them wheatgrass tablets. Wheatgrass has many virtues, and for some, it does help raise hemoglobin levels. Even if it doesn't, it is still quite beneficial for the immune system and skin health. teaching them to enjoy fresh fruit and veggies at a young age can also be a big help, as the vitamins, minerals and antioxidants in fresh foods are greatly needed by the body. Anytime they want fruit like berries or grapes, allow them to indulge themselves. There is nothing better.
And when you do get the report on the younger one, do post it. It is very early and the HbF level will still be high from birth but the comments on the report may be enlightening.
I am inclined to believe that the older boy's condition will manifest as thal intermedia, but it is probably much too early to assess if transfusions will be needed later on.
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dear andy,
I stil hope he's just thal minor. based on the result, is there any chances that he might be just a carrier?
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Joyce,
If the electrophoresis results are accurate, and this would have to be a tremendous mistake for a test to show no HbA, the boy can't be just a minor. However, the extremely high HbF level is keeping his Hb at a minor's level right now, so it is difficult to predict how things will go as he gets older.
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Thanks andy,
im thinking of giving my 1st son hydroxurea, at his age what is the dosage and could i give without doctor's prescribe? actualy, i havn't show the result of my 1st son to the govt doctor as i done the test at private clinic. its not that i dnt trust the govt doctor but from where i come from, the procedure of each treatment taken almost hundred years of time before they decide somting on the patients. im afraid they'll run many tests on my son then by the end of the day, they'll just simply said, come back later after 3/4 months from know. .so i think i want to give a try on hydroxurea witout consulting the doctor first. what say you on tht andy? what's the dosage and rules when he take it ? should he take it alone or together with another supplements?
thanks again andy!
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Hi andy,
im also thinking of giving them resevatrol. cn i give it to my son without consulting the doctor first? if can, please state the dosage taken at thier age.
thnks in advanced-joyce.
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Hi Joyce,
I cannot recommend taking any drug without consulting a doctor, so I cannot recommend hydroxyurea, except under a doctor's supervision. However, resveratrol is a safe, natural supplement and can be taken without a doctor's approval.
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Hi andy,
I want to ask for the specific dosage and vitamin/supplement needed for my 7months thallasemia beta major. At the moment he is on folic acid and multivitamin (Appetton). I’ve visited the puritan site and found out too many vitamins/ supplement there. Please specify the dosage should he be taken at his age. For instance: resveratrol 1000IU/500mg/etc. so do the vitamin E and D, and the IP6 so that I knew the right supplement and dosage for him. And also, some of the supplement is in capsul or softgel form, how can I measure the dosage ?
Im also planning to give his older brother some supplement to help inducing or at least sustain his HbF.(he was never been transfused so far) Please state the right supplement/vitamins together with the dosage should be taken. He is only 15moths.
Thanx!
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Joyce,
I am planning to do a post on what I would give a thal child as far as supplements are concerned. Right now, I would say 500-1000 mcg (.5-1 mg) folic acid daily. !00 IU natural vitamin E daily for the older and every other day for the younger. IP6 capsules can be opened and mixed with liquid (no milk or protein products) or applesauce and used that way. One capsule daily for both kids is perfectly safe. This link shows resveratrol capsules. It may be possible to cut them open to mix the contents with food and taken this way. One capsule every other day for the younger and one every day for the older. http://www.puritan.com/resveratrol-053/resveratrol-250-mg-027980?NewPage=1
Vitamin D should be used if they do not get regular access to the sun. At this age, 400 IU daily would be enough.
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Thanx Andy,
Really helpful. Know i'm ready to order the online supplement that u guys mention about.
Thnx again with the prescription.